69 years old female presented with pallor and generalized lymphadenopathy for 1 month. Hemogram showed Hb: 7.9 g/dl, TLC: 13,200/cumm and Platelet count: 50,000/cumm; DLC: N66 L19, M6, Atypical Plasmacytoid Cells 09. Peripheral smear shows rouleaux formation. Serum LDH, uric acid and creatinine were mildly elevated (297 U/L, 8.3 mg/dl and 1.2 mg/dl respectively) and serum calcium was reduced (3.55 mg/dl). PET-Scan showed metabolically active supra/infra diaphragmatic lymphadenopathy with extranodal and splenic involvement and diffusely increased tracer uptake in bone marrow along with mild bilateral pleural effusion. In view of generalized lymphadenopathy, a provisional diagnosis of Non Hodgkins Lymphoma was considered, and further work-up was advised for confirmation. Cervical lymph node biopsy showed effacement of nodal architecture with large-sized neoplastic lymphoid cells in a background of histiocytes, lymphocytes and plasma cells. On immunohistochemistry, these atypical lymphoid cells were positive for CD3, CD4, CD5, CD30 and TIA-1 and negative for ALK, EMA, CD8, PD-1, CD20, CD10 and EBER-ISH with proliferative index of around 70% (Fig. 1). A diagnosis of ALK-Negative Anaplastic Large Cell Lymphoma was made. Bone marrow evaluation as part of staging work-up revealed 50% atypical plasmacytoid cells with round, central to eccentric nucleus, deeply blue cytoplasm and inconspicuous nucleoli. Hypercellular bone marrow biopsy showed interstitial as well as diffuse infiltration with plasmacytoid cells including atypical and immature forms along with two to three nodular intertrabecular lymphoid aggregates. On IHC, the plasma cells were strongly and diffusely positive for CD138 with clonal restriction for lambda light chain (Fig. 2). The lymphoid aggregates were positive for both CD3 and CD20 suggestive of their reactive nature. In view of negative expression of CD 20 and CD30 in the atypical plasmacytoid cells, the possibility of other differentials such as lymphoplasmacytic lymphoma and small cell variant of anaplastic large cell lymphoma were virtually ruled out. Serum protein electrophoresis showed M band in gamma and beta-2 region. Hence, a final diagnosis of synchronous ALK-Negative ALCL with Plasma cell Myeloma was made. The patient died due to tumor lysis syndrome within next few days.
Fig. 1.
a Lymph Node Biopsy-H &E shows infiltration with large-sized neoplastic lymphoid cells in a background of histiocytes, lymphocytes and plasma cells. b On IHC, CD30 is positive in neoplastic cells. c CD4 (strong and diffuse positive). d ALK-1 is negative
Fig. 2.
a Peripheral smear shows rouleaux. b Bone Marrow aspiration and c Biopsy shows diffuse infiltration with plasmacytoid cells including atypical and immature forms. d On IHC, strong and diffuse positivity for CD138 is noted. e There is clonal restriction for Lambda Light Chain. f Kappa Light Chain is negative
Few published studies have shown plausible biological relationship between B cell lymphomas occurring synchronously or metachronously with multiple myeloma [1–3]. Because of uniquely different cell lines of the two lymphoid malignancies, metachronous occurrence of primary T cell lymphoma and myeloma has been infrequently reported [4–6] based on the hypothesis that the malignant plasma cells evolved under the sustained inducer stimulus of neoplastic T-cells, with retained immunoregulatory functions. It is believed that monoclonal proliferation of Helper-T cells cooperates with cytokines derived from different expression of T cells in the selection of plasma cell clone, resulting in malignant transformation of B cells [7]. However, synchronous occurrence of Anaplastic Large T-cell Lymphoma with Myeloma is very rare without any inciting event as seen in the present case. Reporting such cases will encourage study of potential relationship of immunoregulatory aberrations caused by primary T lymphoid tumors.
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